Patients with progressive nonfluent aphasia (PNFA) can become mute early in the course of the disease. Voxel-based morphometry showed that PNFA is associated with left anterior insula and inferior frontal atrophy. In PNFA with early mutism, volume loss was more prominent in the pars opercularis and extended into the left basal ganglia. Damage to the network of brain regions involved in both coordination and execution of speech causes mutism in PNFA.

INTRODUCTION:Behavioral variant Frontotemporal Dementia (bvFTD) may present sporadically or due to an autosomal dominant mutation. Characterization of both forms will improve understanding of the generalizability of assessments and treatments.METHODS: 135 sporadic (s-bvFTD; mean age 63.3 years; 34% female) and 99 familial (f-bvFTD; mean age 59.9; 48% female) bvFTD participants were identified. F-bvFTD cases included 43 with known or presumed C9orf72 expansions, 28 with known or presumed MAPT mutations, 14 with known GRN mutations, and 14 with a strong family history of FTD but no identified mutation.RESULTS: F-bvFTD were younger and had earlier age of onset. S-bvFTD had higher total NPI-Q scores due to more frequent endorsement of depression and irritability.DISCUSSION: Familial and sporadic bvFTD cases are clinically similar, suggesting the generalizability of novel biomarkers, therapies, and clinical tools developed in either form to the other.

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